Like any great novel, my first post didn’t encompass my entire journey with XLP. There are a few bits of information I had to leave out, however I am now able to tell that part of the story.
I have started writing this many times and never quite found the right words.
This part of my story starts immediately after my transplant. Things didn’t seem to be quite normal, as doctors started to be concerned with some of the results of the tests they were running. None of the tests were coming back as they should or as doctors expected them to come back as after such a transplant. At first, the hope was that it was all just a mistake or a blip. But soon after, we received news of an error with the administration of my transplant: the amount of one of the chemotherapy drugs I was given was incorrect. The name of this drug is Busulfan, and it is commonly used to prep patient’s for a bone marrow transplant. Busulfan is typically administered as a pill, but I was part of study looking at IV based Busulfan. What ended up happening was that someone at the hospital incorrectly titrated the drug, leading to me receiving a reduced dosage. As a result, not all of my bone marrow was killed. Typically, all of a patient’s own bone marrow needs to be killed as to accept the full amount of donor’s bone marrow. So, when I received my transplant the donor cells couldn’t fully take hold in my body. This is known as a mini transplant, something that is used for other illnesses. However this was never used before for XLP and turned out to be a large concern.
As you can probably imagine, this worried both my doctors and parents immensely. The probability of having to get a second transplant in my life seemed highly probable at this point in my life. Additionally, there was the probability of a possible ineffectiveness of my donors cells to fight illness. I was followed incredibly close for many years after by medical professionals. Typically, a patient who received a bone marrow transplant at this point in time may be seen 2-3 times a year, but I was still going monthly to be monitored. Their main concern was how well the donor cells would fight off infections and illness. I had and still have many test done to measure the effectiveness of the donors cells. One of the scariest tests of the donor cells effectiveness for me was when I got Mono. Mono is known to kill a lot XLP patients who haven’t received a transplant in a matter of days. So you can imagine how scary this was when my regular blood test results came back positive for mono. Though as you can probably gather, I survived, surprisingly with no issues at all. Throughout all of this, I was training full time as a paddler, although I drank way too much coffee to compensate for the extreme tiredness I felt because of Mono. Overall this error has had only minor physical effects on how illness affects me, such as the fact that I get sick a little easier and things tend to get worse if I do not really take care of myself. One such example is, if I don’t do a good job taking care of myself, the common cold will turn into bronchitis regularly.
The one area of my life this error has effected greatly is my mental health. Since I can remember, I have always wanted to be as close to a hospital as possible in case something happened. This fear only worsened when I found out about the error from many years ago (my parents did not tell me until I was around 12). Since then, the uncertainty of many things has made my mental health suffer. The reason I ended up wanting to write this now is because of Covid 19 and how this pandemic has reminded me of my struggles with XLP and mental health through the years. The constant extra precautions we are all having to take at the moment, but particularly those with compromised immune systems, are very similar to those I had to take as a young child and throughout my life since. They’ve always and now again have a wearing effect on my mind and body. However I have been very lucky to always have amazing support from my family, friends and girlfriend.
To end this post on a happier note, this mistake has had its benefits as well. For example, Graft vs. Host disease, an illness where your body rejects the donor cells and attacks the donor cells or vice versa, where your donor cells reject you and attack you, has never been an issue for me. This is in part due to my donor being a close family member, but also probably has to do with not receiving 100% of their bone marrow cells. I also had less side effects than those seen by individuals who have had full scale bone marrow transplants, and was able to have an extremely competitive and accomplished paddling career as well as live a more normal life than most.
To this date I still haven’t needed another transplant and may never need one. All of my important tests have been stable for at least 12 years now. I am one unlucky and lucky sun of a gun, all at the same time.
Aaron W. 2020
My Xlp Story 